Diagnosing Sjögren’s disease can be challenging because the disease presents differently from person to person and often overlaps with other autoimmune conditions. While dry eyes and dry mouth are the hallmark symptoms, many patients also experience fatigue, joint pain, nerve involvement, lung disease, or other systemic complications. Because there is no single “gold standard” diagnostic test, clinicians must evaluate a combination of symptoms, laboratory findings, and objective testing to reach an accurate diagnosis.
Why Sjögren’s Is Often Difficult to Diagnose
Sjögren’s is sometimes referred to as a “complex” or “chameleon” disease because its symptoms can mimic other autoimmune illnesses such as rheumatoid arthritis, lupus, or systemic sclerosis. Some patients may initially be diagnosed with another condition before Sjögren’s is recognized. In other cases, patients may have only one dominant symptom for years before additional signs of the disease emerge.
Earlier studies suggested it could take many years for patients to receive a diagnosis, although awareness campaigns and improved education among healthcare professionals have helped shorten that timeline in recent years.
Several factors can contribute to delayed diagnosis, including:
- Symptoms overlapping with other autoimmune diseases
- Mild or fluctuating dryness symptoms
- Negative blood tests despite active disease
- Variability in how Sjögren’s presents between patients
- Lack of access to specialized testing
Clinical Diagnosis Versus Classification Criteria
A key point emphasized in the article is the difference between a clinical diagnosis and research classification criteria.
A clinical diagnosis is made by a healthcare provider using their professional judgment after considering:
- Patient symptoms
- Medical history
- Physical examination
- Bloodwork
- Eye and saliva testing
- Imaging or biopsy results when needed
Classification criteria, on the other hand, were originally designed for research studies to ensure researchers were studying similar patient populations. These criteria are more rigid and may not capture every patient seen in clinical practice.
This means that some individuals may clearly have Sjögren’s from a clinical perspective even if they do not fully meet formal research classification criteria.
Current Classification Criteria for Sjögren’s
The most widely used criteria today are the 2016 ACR-EULAR classification criteria. These criteria combine objective testing and laboratory findings to identify patients with Sjögren’s.
The criteria may include:
- Positive anti-Ro/SSA antibodies
- Abnormal tear production testing (Schirmer’s test)
- Ocular staining tests that assess eye surface damage
- Reduced saliva production
- Minor salivary gland biopsy findings
A patient is classified as having Sjögren’s when enough weighted criteria are met.
Understanding Diagnostic Testing
There are several important diagnostic tools used in evaluating Sjögren’s disease. One common office-based test is the Schirmer’s test, which measures tear production by placing small strips of paper under the lower eyelids. Ocular surface staining may also be performed by an eye specialist to identify damage caused by dryness.
Saliva production can also be measured through salivary flow testing. In some cases, imaging studies such as ultrasound of the salivary glands may help identify inflammation or structural changes associated with Sjögren’s.
When additional confirmation is needed, a minor salivary gland biopsy may be recommended. This procedure usually involves removing tiny salivary glands from the inside of the lower lip for microscopic examination. Lip biopsies are generally considered safe and can provide valuable information when blood tests are negative or symptoms are unclear.
The Role of Blood Tests
Blood tests can provide important clues, but they are not definitive on their own. Common antibodies associated with Sjögren’s include:
- ANA (antinuclear antibody)
- Rheumatoid factor (RF)
- Anti-Ro/SSA
- Anti-La/SSB
However, no single blood test can confirm or rule out Sjögren’s. Some patients with Sjögren’s test negative for common antibodies, while others may test positive for antibodies associated with different autoimmune diseases. Physicians must therefore interpret laboratory findings within the broader clinical picture.
Moving Diagnosis Into Everyday Clinical Practice
Experts continue working toward making Sjögren’s assessment more practical in everyday healthcare settings. A stepwise approach is often recommended, beginning with less invasive screening tools before moving to specialized testing if needed.
Simple office-based assessments may include:
- Standardized symptom questionnaires
- Tear production measurements
- Saliva flow testing
If suspicion remains high, patients may then be referred for additional evaluation such as ocular staining exams, ultrasound imaging, or salivary gland biopsy.
The Importance of Clinical Judgment
Ultimately, diagnosing Sjögren’s requires careful clinical judgment and awareness of the disease’s many presentations. While classification criteria help standardize research, experienced clinicians must evaluate the whole patient rather than relying on a single test result.
The overall message is clear: earlier recognition, increased awareness, and improved access to diagnostic testing are essential to reducing delays in diagnosis and improving outcomes for people living with Sjögren’s.
*summary courtesy of The Sjögren's Book, Fifth Edition, Daniel J. Wallace, MD
